Pulmonary Hypertension

Short phrase for the image: Is pulmonary hypertension curable? Get all the information about this disease: causes, symptoms, and treatments.

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Symptoms and Causes

Pulmonary hypertension refers to an abnormal increase in blood pressure in the pulmonary arteries. This pressure level places an extra burden on the heart, which has to work harder than usual to pump blood to the lungs. Pulmonary hypertension is diagnosed when the mean pulmonary artery pressure at rest is equal to or greater than 25 mmHg.

Based on the origin of the condition, pulmonary hypertension is classified into five types:

According to left ventricular filling pressure values, it is classified as:

  • Precapillary pulmonary hypertension: Normal filling pressure, with a value equal to or less than 15 mmHg. This includes groups 1, 3, 4, and some cases of group 5.
  • Postcapillary pulmonary hypertension: Filling pressure greater than 15 mmHg. This includes group 2 and some cases of group 5.

Symptoms

Pulmonary hypertension symptoms develop slowly and may go unnoticed for months or even years. They worsen as the disease progresses. The most common symptoms include:

  • Dyspnea: Shortness of breath that initially occurs during exertion and later at rest.
  • Syncope during or after exertion.
  • Chest pain.
  • Fatigue.
  • Rapid heart rate and palpitations.
  • Peripheral edema: Swelling due to fluid retention in the ankles and legs.

In the advanced stages of severe pulmonary hypertension and heart damage, the following symptoms appear:

  • Jugular vein distention: Prominent jugular vein in the neck.
  • Cyanosis: Bluish skin discoloration due to lack of oxygen in the blood.
  • Clubbing: Painless deformity of the fingertips and nails.
  • Ascites: Fluid accumulation in the abdomen.

Causes

Blood pressure increases because the arterial walls narrow, become inflamed, and thicken, slowing blood flow to the lungs. This arterial deterioration has various causes depending on the type of pulmonary hypertension:

Risk Factors

Risk factors that increase the likelihood of developing pulmonary hypertension include:

  • Age: The risk increases with aging, though idiopathic pulmonary hypertension is more common in young adults.
  • Family history of pulmonary hypertension or clotting disorders.
  • Overweight.
  • Smoking.
  • Use of certain medications: weight-loss drugs, chemotherapy agents, and stimulants such as amphetamines or cocaine.
  • Exposure to asbestos or specific parasites.
  • Consumption of adulterated rapeseed oil.
  • Blood clotting disorders.

Complications

Possible complications of pulmonary hypertension include:

  • Cor pulmonale: Enlargement and thickening of the right ventricle due to increased workload (the ventricle dilates to hold more blood).
  • Right heart failure: Occurs when the right ventricle's compensation is insufficient for normal heart function.
  • Blood clots in the pulmonary arteries.
  • Pulmonary hemorrhage, which can be fatal.
  • Sudden death due to arrhythmias.
  • In pregnant women, pulmonary hypertension poses a life-threatening risk to both the mother and the baby.

Prevention

Preventing pulmonary hypertension involves managing its risk factors:

  • Maintaining a healthy weight.
  • Not smoking.
  • Avoiding stimulant drugs, certain medications, and adulterated rapeseed oil.

What Specialist Treats Pulmonary Hypertension?

Pulmonary hypertension is diagnosed and treated by specialists in Medicine and Cardiovascular Surgery.

Diagnosis

The diagnosis of pulmonary hypertension is often delayed, as the initial symptoms are common to several cardiac and pulmonary conditions. The diagnosis is confirmed through a range of tests performed to determine the underlying cause of the hypertension and assess its severity:

  • Chest X-ray: an X-ray imaging study that may reveal signs of pulmonary hypertension, such as arterial enlargement, reduced peripheral vascularity, and an enlarged heart.
  • Electrocardiogram (ECG): records the electrical activity of the heart and detects abnormalities in heart rate and rhythm.
  • Echocardiogram: produces images of the beating heart, allowing assessment of blood flow and cardiac pumping function. It can help rule out heart diseaseHeart DiseaseHeart Disease as the cause of the hypertension.
  • Pulmonary function testing and arterial blood gas analysis: involves breathing into a spirometer to measure the volume of air the lungs can hold and the amount of air that can move in and out of the lungs.
  • Exercise stress test: evaluates cardiac and pulmonary function during physical exertion.
  • Blood tests: identify markers of infection or coagulation disorders, antibodies associated with connective tissue diseases, and indicators of liver and thyroid function.
  • Ventilation-perfusion (V/Q) scan: an imaging study used to assess airflow and blood circulation within the lungs. It helps rule out thromboembolism as the underlying cause.
  • Right heart catheterization (pulmonary hemodynamic study): a catheter is guided through a blood vessel into the right ventricle and pulmonary artery to measure pulmonary arterial pressure. This test confirms the diagnosis of pulmonary hypertension and provides information about its severity.
  • Computed tomography (CT) scan: this imaging test is used to identify pulmonary conditions such as COPD or pulmonary fibrosis.

Treatment

Treatment is focused on improving symptoms, preventing disease progression, and prolonging survival, as pulmonary hypertension has no cure. Common treatment options include the following:

  • Pharmacological treatments: help reduce both symptoms and the risk of developing complications.
    • Vasodilator medications: widen the arteries and increase blood flow, reducing pressure within the lungs.
    • Anticoagulant medications: help prevent the formation of blood clots in the lungs.
    • Digoxin: increases the strength of cardiac contractions, allowing the heart to pump more blood.
    • Diuretics: promote the elimination of excess fluid from the body.
    • Oxygen therapy: administered when hypoxemia (low blood oxygen levels) is present. It helps reduce pulmonary vascular resistance.
  • Surgical procedures: when medication does not provide the expected results, surgical intervention may be necessary.
    • Atrial septostomy: an opening is created between the left and right atria to allow blood to flow between them. This reduces pressure on the right side of the heart and improves left cardiac output.
    • Pulmonary endarterectomy: an open surgical procedure performed to remove blood clots from the pulmonary arteries.
    • Balloon pulmonary angioplasty: a small balloon is introduced into the blood vessel through a catheter and inflated to help break up obstructions. This procedure is used when open surgery is not feasible.
    • Lung or heart transplantation: considered in very advanced stages of the disease when no other treatment options are effective.
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